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Juvenile Systemic Lupus Erythematosus (JSLE) is a subset of SLE, with onset before eighteen years of age and associated with increased mortality and morbidity. JSLE is a complex autoimmune disease with respect to its underlying genetics. It is characterised by the disruption of immune tolerance, resulting in cascade of immune responses including the Th2 response with production of interleukins (ILs) 4,5,10, as seen with allergic disorders. Along with this, polyclonal activation of B lymphocytes results in the production of autoantibodies, particularly of the IgG, IgM and antinuclear IgE autoantibodies. JSLE is frequently recognized in girls. The purpose of this case report is to share an uncommon presentation of JSLE in a 8-year-old boy who was diagnosed as a case of atopic eczema at the age of 8 months and later on, at the age of 4 years, he was presented with the case of full blown JSLE with early development of stroke within one year of diagnosis of JSLE.
Subject(s)
Dermatitis, Atopic , Lupus Erythematosus, Systemic , Humans , Male , Lupus Erythematosus, Systemic/diagnosis , Lupus Erythematosus, Systemic/immunology , Lupus Erythematosus, Systemic/complications , Child , Dermatitis, Atopic/diagnosis , Dermatitis, Atopic/immunologyABSTRACT
Erythrokeratodermia variabilis (EKV) is a rare inherited genodermatosis characterised by migratory and erythematous patches changing over the course of hours to days and fixed keratotic plaques. The disease begins mostly at birth or within the first year of life; it very rarely starts after childhood. We present here a sporadic case with adult onset EKV that was aggravated by pregnancy with spontaneous resolution, and later on, after crash diet more persistent patches and plaques appeared with no spontaneous resolution. The patient showed excellent response to systemic retinoids.
Subject(s)
Erythrokeratodermia Variabilis , Humans , Female , Pregnancy , Adult , Erythrokeratodermia Variabilis/genetics , Pregnancy ComplicationsABSTRACT
Objective: To assess the current trends in the field of artificial intelligence in medicine by analysing 100 most cited original articles relevant to the field. METHODS: The bibliometric analysis was conducted in September 2022, and comprised literature search on Scopus database for original articles only. Google and Medical Subject Headings databases were used as resources to extract key words. In order to cover a broad range of articles, original studies comprising human as well as non-human subjects, studies without abstract and studies in languages other than English were part of the inclusion criteria. There was no specific time period applied to the search and no specific selection was done regarding the journals in the database. The screening was done using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines to extract the top 100 most cited articles in the field of artificial intelligence usage in medicine. Data was analysed using SPSS 23. RESULTS: Of the 11,571 studies identified, 100(0.86%) were analysed in detail. The studies were published between 1986 and 2021, with a median of 43 citations (IQR 53) per article. The journal 'Artificial Intelligence in Medicine' accounted for the highest number 9(9%)) of articles, and the United States was the country of origin for most of the articles 36(36%). Conclusion: The trends, development and shortcomings in field of artificial intelligence usage in medicine need to be understood to conduct an effective research in areas that still need attention, and to guide the authorities to direct their funding accordingly.
Subject(s)
Artificial Intelligence , Bibliometrics , Clinical Medicine , Humans , Periodicals as Topic/statistics & numerical dataABSTRACT
Folliculotropic mycosis fungoides (FMF) is a variant of mycosis fungoides characterized by infiltration of hair follicle epithelium by neoplastic lymphoid cells. Generally, it is usually typified by indurated plaques and tumours mainly on the head and neck. However, a wide range of clinical signs have been noted. The clinical presentation of FMF may include prurigo-like lesions, acneiform lesions, cysts, nodules, areas of scarring alopecia, and hypopigmented plaques or papules with follicular prominences. The average age of diagnosis is 60 years while it is rare in childhood and adolescence. We discuss the case of a 12-year-old male patient who had an asymptomatic, erythematous, infiltrating plaque across his left nasolabial fold for three months prior to presentation. Histological assessment of lesion showed characteristic findings of follicular mucinosis with predominance of CD4+ lymphocytes and immunohistochemical studies were positive for CD3+ stains. An increased CD4:CD8 ratio and negative CD20 was also shown. Both findings were consistent with diagnosis of FMF.
Subject(s)
Mycosis Fungoides , Skin Neoplasms , Male , Adolescent , Humans , Child , Middle Aged , Skin Neoplasms/diagnosis , Skin Neoplasms/pathology , Mycosis Fungoides/diagnosis , Mycosis Fungoides/chemistry , Mycosis Fungoides/pathology , Hair Follicle/chemistry , Hair Follicle/pathologyABSTRACT
OBJECTIVE: To compare the efficacy of platelet-rich plasma (PRP) versus 5% topical minoxidil for the treatment of androgenetic alopecia (AGA). STUDY DESIGN: Randomised-controlled trial. Place and Duration of the Study: Department of Dermatology, PNS Shifa Hospital, Karachi, Pakistan, from 1st November 2021 to 31st July 2022. METHODOLOGY: Seventy AGA patients aged between 18-60 years of either gender were randomly divided into two groups. Group A was given 5% topical minoxidil and Group B was given PRP. Both groups were followed up over a period of 6 months, and the final analysis was done with the help of global photography, hair pull test, and patient satisfaction score. RESULTS: At the end of 6th month, 27 patients (77%) in Group A had a negative hair pull test as compared to only 14 (40%) in Group B (p = 0.001). In Group A, 32 patients (91.4%) reported improvement in hair scalp from baseline. Whereas, in Group B, 26 patients (74.3%) reported improvement from baseline (p = 1.00). PRP was effective in 26 patients (74.5%) and 5% topical minoxidil in 15 patients (43.7%) (p = 0.007). CONCLUSION: PRP therapy can be a useful alternative to topical minoxidil in the treatment of AGA. KEY WORDS: Androgenetic alopecia, Global photography, Platelet-rich plasma, 5% Topical minoxidil, Treatment.
Subject(s)
Minoxidil , Platelet-Rich Plasma , Humans , Adolescent , Young Adult , Adult , Middle Aged , Minoxidil/therapeutic use , Alopecia/drug therapy , Hair , HospitalsABSTRACT
SJS/TEN (Stevens-Johnson syndrome/toxic epidermolysis necrosis) is a T-cell mediated hypersensitivity syndrome in which cytotoxic CD8+ cells react against keratinocytes, resulting in widespread apoptosis and cell necrosis. About 90% of these cases are attributed to drug reactions, while 10% are idiopathic. The disease is classified according to body surface area (BSA) involvement and the thickness of epidermal loss. We report a case of a female with borderline personality disorder on antipsychotic medication, who developed SJS/TEN overlap after taking ciprofloxacin for her urinary tract infection (UTI). Her condition improved with meticulous management, but after switching her antibiotic from intravenous clarithromycin to oral linezolid, she developed SJS/TEN again, this time with more severe involvement. She received active management involving a multidisciplinary approach. Her condition improved slowly and, after one month, her lesions began to heal, and she was discharged with advice not to use both antimicrobial drugs in the future.
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Introduction The majority of nail diseases in children are comparable to those in adults, while there are some physiological changes that start to happen around this age and go away over a few years. These conditions could be symptoms of infections and systemic illnesses. Pediatric nail disorders are typically easy to diagnose clinically, although there are occasionally conditions that masquerade as juvenile nail problems. Dermoscopy has grown in favour as a rapid, easy, non-invasive clinical procedure for examining nail diseases. This study aims to assess dermoscopic findings of child nail diseases. Methods A prospective analysis was conducted for seven months between January and July, 2022, at PNS Shifa Hospital, Karachi, Pakistan. A total of 180 patients who presented in the outpatient department with any dermatological complaint underwent thorough history and examination. Special emphasis was given to clinical and dermoscopic examinations of nails. Data analysis enclosed descriptive and inferential statistics. The quantitative data was presented with help of mean and standard deviation, while the qualitative data was presented with help of frequency and percentage tables. Chi-square test was applied to compare nail findings diagnosed on clinical examination and dermoscopic examination. Results The mean age of study subjects was 9.4±3.2 years and ranged between five years and 18 years. The most common nail changes were hand eczema (n=41, 23%) followed by nail changes due to nutritional disorders (n=38, 21%), anaemia (n=34, 19%) and habit tic deformity (n=31, 17.2%). Conclusions Clinical evaluation is crucial for nail disease diagnosis. Dermoscopy of nails can help with the final diagnosis of nail disease and confirm clinical diagnoses. Also, it aids in the management of nail illnesses by providing a clearer picture of pathology and nail structure.
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Nivolumab is a human immunoglobulin G4 (IgG4) monoclonal antibody that binds to the PD-1 receptor on T-cells and blocks its interaction with PD-L1 and PD-L2, releasing the PD-1 pathway-mediated inhibition of the immune response, including the anti-tumor immune response, resulting in decreased tumor growth. Here, we present a case of a 56-year-old lady with a diagnosis of squamous cell carcinoma (SCC) of the lip who presented with dystrophy of 20 nails, distal onycholysis, yellow-black discoloration of nail plates, painful paronychia with superimposed bacterial infection of big toes of both feet for three months. Few warty growths were also appreciated on big toes of both feet. She had undergone for her SCC 33 sessions of radiotherapy and 43 cycles of nivolumab 140mg for 60 minutes every two weeks. Following discontinuing this drug, the peri-ungual and nail bed inflammation improved, however nail plate dystrophy persisted. To our knowledge, the occurrence of nail dystrophy with nivolumab has never been reported before but it has been described with other targeted therapies.
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This study aimed to examine the current situation of anti-cancer drug shortages in Pakistan, namely its determinants, impacts, adopted mitigation strategies, and proposed solutions. Qualitative semi-structured, in-depth interviews were conducted with 25 pharmacists in oncology hospitals in Pakistan from August to October 2021. Data were collected in person and online, recorded, and subjected to inductive thematic analysis after being transcribed verbatim. Most participants experienced anti-cancer drug shortages that increased during the pandemic. Etoposide, paclitaxel, vincristine, dacarbazine, and methotrexate were frequently short. Important causes included the compromised role of regulatory authorities, lack of local production, and inventory mismanagement. The impacts were delayed/suboptimal treatment and out-of-pocket costs for patients, patients' prioritization, increased workload, negative work environment, and patients' trust issues for pharmacists. The participants proposed that a cautious regulator's role is needed to revise policies for all stakeholders and support all stakeholders financially at their level to increase access to these medicines. Based on the outcomes, it is clear that anti-cancer medicine shortages are a current issue in Pakistan. Governmental authorities need to play a role in revising policies for all levels of the drug supply chain and promoting local production of these drugs. Stakeholders should also collaborate and manage inventory.
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Antineoplastic Agents , Neoplasms , Humans , Pharmacists , Qualitative Research , Neoplasms/drug therapy , Patients , Antineoplastic Agents/therapeutic use , PakistanABSTRACT
Drug shortage is a global issue affecting low, middle, and high-income countries. Many countries have developed various strategies to overcome the problem, while the problem is accelerating, affecting the whole world. All types of drugs, such as essential life-saving drugs, oncology medicines, antimicrobial drugs, analgesics, opioids, cardiovascular drugs, radiopharmaceutical, and parenteral products, are liable to the shortage. Among all pharmaceutical dosage forms, sterile injectable products have a higher risk of shortage than other forms. The causes of shortage are multifactorial, including supply issues, demand issues, and regulatory issues. Supply issues consist of manufacturing problems, unavailability of raw materials, logistic problems, and business problems. In contrast, demand issues include just-in-time inventory, higher demand for a product, seasonal demand, and unpredictable demand. For regulatory issues, one important factor is the lack of a unified definition of drug shortage. Drug shortage affects all stakeholders from economic, clinical, and humanistic aspects. WHO established global mitigation strategies from four levels to overcome drug shortages globally. It includes a workaround to tackle the current shortage, operational improvements to reduce the shortage risk and achieve early warning, changes in governmental policies, and education and training of all health professionals about managing shortages.